Department of child health care, The Children's Hospital of Zhejiang University School of Medicine, 57# zhugan road, Hangzhou, Zhejiang Province, China.
BMC Pediatr. 2014 Apr 4;14:90. doi: 10.1186/1471-2431-14-90.
Williams syndrome (WS) is a neurodevelopmental disease characterized by compelling psychological phenotypes. The symptoms span multiple cognitive domains and include a distinctive pattern of social behavior. The goal of this study was to explore adaptive behavior in WS patients in China.
We conducted a structured interview including the Infants-Junior Middle School Students Social-life Abilities Scale in three participant groups: children with WS (n = 26), normally-developing children matched for mental age (MA, n = 30), and normally-developing children matched for chronological age (CA, n = 40). We compared the mean scores for each domain between the three groups.
Children with WS had more siblings than children in the two control groups. The educational level of the caregivers of WS children was lower than that of the control children. We found no differences in locomotion, work skill, socialization, or self-management between the WS and MA groups. WS children obtained higher scores of self-dependence (df = 54, Z = -2.379, p = 0.017) and had better communication skills (df = 54, Z = -2.222, p = 0.026) compared with MA children. The CA children achieved higher scores than the WS children for all dimensions of adaptive behavior.
WS children have better adaptive behavior skills regarding communication and self-dependence than normal children matched for mental age. Targeted intervention techniques should be designed to promote social development in this population.
威廉姆斯综合征（WS）是一种神经发育疾病，其特征是具有强烈的心理表现型。其症状涉及多个认知领域，包括独特的社会行为模式。本研究的目的是探索中国 WS 患者的适应行为。
我们对三组参与者进行了结构访谈，包括婴幼儿-初中生社会生活能力量表：WS 患儿（n = 26）、与心理年龄（MA）匹配的正常发育儿童（n = 30）和与实际年龄（CA）匹配的正常发育儿童（n = 40）。我们比较了三组儿童在每个领域的平均得分。
WS 患儿的兄弟姐妹比对照组的儿童多。WS 患儿的照料者的教育水平低于对照组儿童。WS 组与 MA 组在运动、劳动技能、社交和自我管理方面没有差异。WS 患儿的自理能力得分（df = 54，Z = -2.379，p = 0.017）和沟通能力得分（df = 54，Z = -2.222，p = 0.026）高于 MA 组。在适应行为的所有维度上，CA 儿童的得分均高于 WS 儿童。
WS 患儿在沟通和自理方面的适应行为技能优于 MA 正常儿童。应该设计有针对性的干预技术，以促进该人群的社会发展。
